Clinical, radiological, and pathological features of 16 papillary glioneuronal tumors

Abstract

Papillary glioneuronal tumors (PGNTs) are a novel distinct intracranial neoplastic entity. In this study, the authors aimed to analyze the clinical, radiological, and pathological features of PGNT. Clinical charts and radiographs of 16 cases of PGNT surgically treated between 2006 and 2013 were retrospectively reviewed. Follow-up evaluations and a literature review were performed. The study included nine males and seven females with a mean age of 23.8 years. The most common preoperative symptom was headache (68.8 %, 11 of 16 patients). Radiological results showed that the frontal lobe (25.0 %) was the most common portion of the brain involved, and 13 lesions (81.3 %) presented with cystic appearance with or without solid elements. All patients were misdiagnosed as non-PGNT tumors. Complete resection was achieved in 12 patients (75.0 %). Ki67 staining was positive in <1 % of cells present in eight lesions and varied in the other eight lesions, with a range of 1 % to 13 %. The mean follow-up duration was 56.2 months, and no recurrence was observed. Seventy-seven PGNTs (40 males, 51.9 %) have been reported, and 49 patients stained positive for Ki67/MIB-1. Of these cases, 28 (57.1 %) had positive staining rates of ≥1 %. In 51 patients for whom outcome information was available, six (11.8 %) exhibited recurrence, and the recurrence rates for complete resection and incomplete resection were 5.1 % and 33.3 %, respectively. PGNTs displayed a wide spectrum of clinical and radiological phenotypes, and they were more frequently observed in the frontal lobe and in young patients without sex predilection. Fair outcomes could be achieved by complete resection. Although PGNT displayed indolent pathobiology, atypical appearances were observed. More patients and longer follow-up periods are needed to further elucidate the biological features of PGNTs.